171 results match your criteria: "Groupe Hospitalier Necker-Enfants Malades[Affiliation]"

Intestinal intussusception survey about diagnostic and nonsurgical therapeutic procedures.

Pediatr Radiol

October 1999

Paediatric Radiology Department, Groupe Hospitalier Necker Enfants-Malades, 149 rue de Sèvres, F-75 743 Paris Cedex 15, France.

Objective: To provide an overview of the diagnostic and therapeutic procedures performed by European paediatric radiologists in the management of intussusception.

Materials And Methods: A postal survey was sent to the European members of ESPR. Items surveyed included diagnostic imaging procedures (plain films, US, contrast enema [CE]), contrast medium used (barium, iodine, air, saline solution), and imaging technique used for monitoring during reduction (films, fluoroscopy, US).

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[Syringomyelia in children].

Neurochirurgie

June 1999

Service de Neurochirurgie Pédiatrique, Groupe Hospitalier Necker Enfants Malades, Paris.

From 1985 to 1997, 442 children were treated for syringomyelia. One hundred and eighty eight had syringomyelia in association with Chiari I malformation. In 65% of the case scoliosis was the initial symptom.

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We report a 5 1/2-year-old boy with juvenile rheumatoid arthritis (JRA) and lower-limb lymphoedema. US, MRI and lymphangiography were performed. Based on the lymphangiographic study, we propose a pathogenesis based on obstruction of normal superficial lymphatic vessels in the affected limb.

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The ability of Ca2+/phospholipid-dependent protein kinase (protein kinase C, PKC) to stimulate cAMP phosphodiesterase (PDE) activity in a liver Golgi-endosomal (GE) fraction was examined in vivo and in a cell-free system. Injection into rats of 4 beta-phorbol 12-myristate 13-acetate, a known activator of PKC, caused a rapid and marked increase in PKC activity (+325% at 10 min) in the GE fraction, along with an increase in the abundance of the PKC alpha-isoform as seen on Western immunoblots. Concurrently, 4 beta-phorbol 12-myristate 13-acetate treatment caused a time-dependent increase in cAMP PDE activity in the GE fraction (96% at 30 min).

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A total of 106 children suffering from perennial rhinitis and/or asthma, and all allergic to Dermatophagoides pteronyssinus (DP), underwent nasal provocation challenge (NPC) with DP to determine the best method of diagnosis. Posterior rhinomanometry was uninterpretable in 17 patients and gave negative results in 31. Clinical scores for sneezing and rhinorrhea were more effective but did not diagnose the disorder in 11 children.

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Spatio-temporal expression of FGFR 1, 2 and 3 genes during human embryo-fetal ossification.

Mech Dev

September 1998

INSERM U 393 and Département de Génétique, Assistance Publique - Hôpitaux de Paris, Groupe Hospitalier Necker Enfants Malades, 149 rue de Sèvres, 75743, Paris Cedex 15, France.

Mutations in FGFR 1-3 genes account for various human craniosynostosis syndromes, while dwarfism syndromes have been ascribed exclusively to FGFR 3 mutations. However, the exact role of FGFR 1-3 genes in human skeletal development is not understood. Here we describe the expression pattern of FGFR 1-3 genes during human embryonic and fetal endochondral and membranous ossification.

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The endosome-lysosome transfer of in vivo internalized insulin and glucagon has been studied in a rat liver cell-free system and compared to that of galactosylated bovine serum albumin (GalBSA), a ligand of the asialoglycoprotein receptor. Density-gradient analysis of a postmitochondrial supernatant isolated 8 min after injection of [125I]iodoinsulin showed that the membrane-associated radioactivity (55% of the total) migrated as a single peak at the position of galactosyltransferase, a Golgi marker (1.08-1.

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We performed a prospective random study to assess possible thyroid stunning by a 185-MBq iodine-131 dose used to diagnose thyroid remnants. Patients with differentiated thyroid carcinoma were included after total or near-total thyroidectomy. They were randomly assigned to two groups.

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[The significance and role of speech therapy in rehabilitation after lip surgery].

Rev Stomatol Chir Maxillofac

November 1997

Service de Stomatologie et de Chirurgie Maxillo-faciale Pédiatrique, Groupe Hospitalier Necker Enfants-Malades, Paris.

Post-operative functional rehabilitation of the orofacial region has greatly benefited from progress in orthophony. Technical know-how in pediatric patients can be adapted to the specific requirements of postoperative rehabilitation in children.

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AL amyloidosis is a rare disease. Its diagnosis is based on histopathology. It is always secondary to light chain, only rarely heavy chain, synthesis of monoclonal immunoglobulin, associated or not with tumoural proliferation (myeloma, Waldenström's macroglobulinaemia).

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[Infantile cystinosis].

Rev Prat

September 1997

Département de pédiatrie médicale, Néphrologie pédiatrique, Groupe hospitalier Necker-Enfants malades, Paris.

Infantile cystinosis is a metabolic lysosomal storage disease of cystine affecting most of the body cells. The first symptoms appear after 5-6 months of life: anorexia, vomiting, polyuria, polydipsia and failure to thrive, associated with the signs of tubular Fanconi syndrome including glycosuria, proteinuria, loss of bicarbonate, phosphate, potassium, sodium, etc. Treatment with cysteamine is effective if started as early as possible.

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Advances in antimitotic treatments have improved the prognosis of cancer in young subjects. The resulting increase in life expectancy raises the question of the subject's future fertility, a question that should be posed before beginning any anticancer therapy which could lead to a gonadal failure. If oocyte donation remains the alternative indication proposed for these patients desiring a child, it is important to assess the tissue alterations in the uterus, to verify its vascularization under suitable treatment and appreciate the other alternative directions.

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Congenital lumbosacral lipomas.

Childs Nerv Syst

June 1997

Department of Pediatric Neurosurgery, Groupe Hospitalier Necker-Enfants Malades, Paris, France.

Congenital lumbosacral lipomas can be responsible for progressive defects. The general feeling is that tethering of roots, filum, or cord probably explains this evolution, and that untethering of these structures could prevent late deterioration. Like the vast majority of neurosurgeons, we too have routinely and systematically operated on lumbosacral lipomas, even in the absence of neurological deficits.

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Background: Spindle-cell hemangioendothelioma is a soft tissue skin tumor recently identified histologically. It can occur at all ages but generally is seen in young adults. The lesion usually occurs as a subcutaneous mass involving the limbs.

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Allergic rhinitis is a common disease in childhood, but nasal cytology is rarely used by pediatricians. We compared two techniques of cell sampling, brushing and blowing the nose, among 77 children suffering from chronic rhinitis, of whom 59 were allergic. Staining by the May-Grunwald-Giemsa method enabled the evaluation of the density of cells and especially differential counting of the inflammatory cells.

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Midazolam (M) is used as an induction agent for anesthesia. The main metabolite is alpha-hydroxymidazolam (OM), which is pharmacologically active. Use of M for sedation is a recent application, rapidly gaining favor.

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The surgical treatment of congenital naevi of the head and neck often require the use of expansion prostheses. The high risk of complications in children such as infection, necrosis, prosthetic exposure, has led the authors to propose a new therapeutic approach, consisting of deferred cutaneous expansion, which uses the skin tension as an expansion motor. This allows repair in one or several surgical phases, of large defect using the skin of the same anatomical unit, with good esthetic results.

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